Update on Cutaneous Soft Tissue Tumors

Dermawan, Josephine K; Ko, Jennifer S.; Billings, Steven D.

doi:10.1016/j.path.2021.03.002

Cited by 6 publications

(9 citation statements)

References 64 publications

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“…Identification of EWSR1::SMAD3 -rearranged fibroblastic tumor is critical to provide prognostic information and guide appropriate therapeutic decisions and most importantly avoid overtreatment ( 9 , 14 , 15 ). When encountered with uniform spindle cells in a collagenous to myxoid stroma, diffuse and strong ERG expression will render a clue for EWSR1::SMAD3 -rearranged fibroblastic tumor.…”

Section: Discussionmentioning

confidence: 99%

EWSR1::SMAD3-rearranged fibroblastic tumor: A case with twice recurrence and literature review

et al. 2022

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EWSR1::SMAD3-rearranged fibroblastic tumor is a recently described entity that mostly occurs in acral locations. Only 15 cases have been reported in the English literature, with a wide age range and marked female predominance. The most common sites are the foot, followed by the hand and the distal lower leg. There are four cases that recurred locally during 5–120 months of follow-up, with no metastases to date. Herein, we presented a case of EWSR1::SMAD3-rearranged fibroblastic tumor that recurred twice in a 20-year-old man. The patient presented with a second recurrent painful nodule in the left plantar of the second toe. Grossly, the lesion was pale solid and well-defined, measuring 9 × 8 × 9 mm in size. Histological examination revealed a monomorphic spindle cell tumor composed of cellular fascicles of bland fibroblasts in a collagenous to myxoid stroma with low mitotic activity, which evoked a wide spectrum of differential diagnoses. Immunohistochemically, the tumor cells were diffusely and strongly positive for ERG while negative for S100, α-SMA, CD34, and other vascular markers. An unbalanced rearrangement of EWSR1 was demonstrated by fluorescence in situ hybridization (FISH), and a gene fusion between EWSR1 exon 7 and SMAD3 exon 6 was confirmed by RT-PCR and Sanger sequencing. This case recurred twice within 6 years with no sign of further relapse and metastasis at another 9-month follow-up since the last surgery, indicating that this tumor was benign but prone to local recurrence. Nevertheless, more cases and further studies are needed to better interpret the biological behavior of this new entity.

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Section: Discussionmentioning

confidence: 99%

EWSR1::SMAD3-rearranged fibroblastic tumor: A case with twice recurrence and literature review

et al. 2022

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“…1 Though originally considered a rare morphologic variant of dermatofibroma/ benign fibrous histiocytoma (BFH), the World Health Organization now considers EFH to be a biologically distinct entity. 1,5 Arguments in favor of their separate classification point to the absence of ALK rearrangements in BFH, although a subset of EFH is ALK negative. 1,4 EFH is most frequently observed in the lower extremities of young to middle-aged adults.…”

Section: Introductionmentioning

confidence: 99%

“…1,5 Arguments in favor of their separate classification point to the absence of ALK rearrangements in BFH, although a subset of EFH is ALK negative. 1,4 EFH is most frequently observed in the lower extremities of young to middle-aged adults. 1,2 The lesion often appears as a solitary exophytic nodule, with histopathology demonstrating an epidermal collarette and pronounced vascularity.…”

Section: Introductionmentioning

confidence: 99%

“…1,4 EFH is most frequently observed in the lower extremities of young to middle-aged adults. 1,2 The lesion often appears as a solitary exophytic nodule, with histopathology demonstrating an epidermal collarette and pronounced vascularity. 5 Head and neck presentation is rare, and no cases of EFH have been reported in the external auditory canal.…”

Section: Introductionmentioning

confidence: 99%

“…Epithelioid fibrous histiocytoma (EFH) is a benign cutaneous fibrohistiocytic tumor that commonly features predominant epithelioid morphology and anaplastic lymphoma kinase (ALK) gene fusion 1 . Though originally considered a rare morphologic variant of dermatofibroma/benign fibrous histiocytoma (BFH), the World Health Organization now considers EFH to be a biologically distinct entity 1,5 . Arguments in favor of their separate classification point to the absence of ALK rearrangements in BFH, although a subset of EFH is ALK negative 1,4 .…”

Section: Introductionmentioning

confidence: 99%

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